Acyl-CoA dehydrogenases (BCAA branch)
Cofactors used
Clinical / pharmacological. MCAD deficiency: hypoketotic hypoglycaemia, dicarboxylic aciduria, SIDS-like presentation
What it does, reaction by reaction
Amino Acid Catabolism Overview (glucogenic vs. ketogenic amino acids; brief overview of branched-chain amino acid catabolism and phenylalanine/tyrosine catabolism as representative examples) Amino Acid & Nitrogen Metabolism · Cytosol + mitochondrion
Isoleucine-derived 2-methylbutyryl-CoA → tiglyl-CoA → 2-methyl-3-hydroxybutyryl-CoA → 2-methylacetoacetyl-CoA → acetyl-CoA + propionyl-CoA
Converts Isoleucine-derived 2-methylbutyryl-CoA into Tiglyl-CoA
› Notes
Dehydrogenase, hydratase, dehydrogenase, and thiolase reactions process the acyl chain; the first dehydrogenation uses FAD and the hydroxyacyl dehydrogenase uses NAD⁺. Propionyl-CoA is converted by propionyl-CoA carboxylase (biotin, ATP, bicarbonate), methylmalonyl-CoA epimerase, and methylmalonyl-CoA mutase (adenosylcobalamin) to succinyl-CoA, so isoleucine is both ketogenic and glucogenic.
Showing all 1 reactions.
What accelerates and inhibits it
Regulation is pathway-specific, so each context is listed separately
No regulation recorded for this enzyme in the source documents.
Recent literature
Europe PMC · fetched just now · sorted by publication date
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1
Integrated transcriptome and metabolome reveal <i>Mycoplasma hyopneumoniae</i> -induced lipid accumulation, inflammation, oxidative stress, and energy imbalance in the liver of pigs.
Xue X, Bu Y, Yu C, Li J, Yang J, Gao M, Wang P, Gan Y, Zhang W, Fan S, Zhou R, Yin Z, Zheng X. · 2026-06-03
open access unreviewed -
2
Lipid Metabolic Reprogramming and Epigenetic Co-Dysregulation Across the Central Chondrosarcoma Grade Spectrum: A Multi-Cohort RNA-seq Study
Ayhan B, Dönmez N, Ayhan Z. · 2026-06-01
open access unreviewed -
3
Analysis of differentially expressed genes related to acute lung injury and their role in metabolic pathways: An integrative study using GEO data.
Yang Y. · 2026-05-01
open access unreviewed -
4
Natural Plant-Derived Compounds Targeting Oxidative Stress and Inflammation in NAFLD-Mechanisms and Repositioning Potential.
Theodorou RE, Vrettos N, Theodosis-Nobelos P. · 2026-04-29
open access unreviewed -
5
Integrated Analysis of Transcriptome and Metabolome Reveals Molecular Responses to Ammonia Stress in the Gills of <i>Litopenaeus vannamei</i> Under Low-Salinity Conditions.
Zhao Y, Ding Y, Zhou F, Hu X, Yang Q, Cao Y. · 2026-04-13
open access unreviewed -
6
Clinical, Biochemical and Molecular Characterisation of Newborns With Fatty Acid β-Oxidation Disorders: Novel Variants in the ACADM , ACADVL and SLC22A5 Genes.
Hidalgo Mayoral I, Herranz Cecilia A, Rodríguez-Jiménez C, Carazo Álvarez A, Bergua Martínez A, Andrade Guerr… · 2025-09-29
unreviewed -
7
Two Years of Expanded Newborn Screening in Russia: High-Throughput Detection of Inherited Metabolic Disorders by Tandem Mass Spectrometry with Next-Generation Sequencing Confirmation.
Zakharova EY, Baydakova GV, Baranova PV, Aleksandrova DY, Shchagina OA, Itkis YS, Milovanova NV, Nagornova TS… · 2026-03-02
open access unreviewed -
8
Activation of IRF3 in cardiomyocytes impairs mitochondrial oxidative function through PGC-1α inhibition and drives heart failure.
Kumari M, Evangelakos I, Deshpande A, De Jong KA, Schmiedel N, Palacio-Escat N, Chaves-Filho AB, Frias-Soler … · 2026-02-27
cited 2× open access unreviewed -
9
A Novel CYP2E1 Inhibitor, 4-Methyl-5-Acetylthiazole (Q11), Alleviates Obesity Via Modulating Adipose Inflammation and Mitochondrial Dysfunction.
Qiu J, Gao L, Wang L, Wang X, Jia L, Deng M, Tang L, Wen Q, Gao N, Xu H, Qiao H. · 2025-12-20
cited 2× open access unreviewed -
10
MetaboMiNR: An Online Tool to Analyze Label-Free Proteomic Experiments With a Focus on Metabolism and Nuclear Receptors.
Saikali MF, Cummins CL. · 2026-01-01
cited 2× open access unreviewed
External claims. These come from an index outside this database and are not checked against it. Treat them as leads.